Central Nervous System Manifestations Research Articles

Para-Infectious Acute Transverse Myelitis Following Sars-Cov2 Infection: A Case Report

Abstract Introduction: The new pandemic has highlighted new ways of clinical presentation of severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) including a possible link to central nervous system (CNS) manifestation. Case report: We present a case of acute transverse myelitis following a SARS-CoV 2 infection in a 34-year-old man who presented with bilateral lower-extremity weakness and acute urinary retention. Cervical magnetic resonance imaging showed a T2 hyperintense signal abnormality at C3-C6 and D3-D7 levels consistent with acute myelitis. Conclusion: SARS-CoV 2 can cause myelitis by immune-mediated mechanisms, therefore it is extremely important for the clinicians to recognise the signs and promptly treat this neurological complication.

Jamestown Canyon and snowshoe hare virus seroprevalence in New Brunswick.

Jamestown Canyon virus (JCV) and snowshoe hare virus (SSHV) are wide-ranging mosquito-borne arboviruses in the California serogroup viruses (CSGV) that are known to circulate in New Brunswick. Despite potential for debilitating central nervous system manifestations, the prevalence of human exposure to these viruses in New Brunswick is unknown. The goal of this study was to quantify rates of human exposure in New Brunswick to these neglected arboviruses. A retrospective, anonymized provincial serosurvey was performed using a stratified random sample of residual sera submitted between May 2015 and August 2016. To determine the seroprevalence of JCV and SSHV, competitive enzyme-linked immunosorbent assay-positive samples were confirmed positive using plaque-reduction neutralization testing (PRNT). A total of 452 serum samples were screened. The seroprevalence of antibodies against CSGV was estimated to be 31.6% (95% CI 27.4% to 36.1%) with 143 positive samples. PRNT results indicated that most single virus exposures were due to JCV (38 of 143; 26.6%) rather than SSHV (3 of 143; 2.1%). The species of CSGV, to which the remaining 102 seropositive people were exposed, could not be precisely determined. The prevalence of human exposure to CSGV is high but comparable to rates observed in other Atlantic Canadian jurisdictions. Studies such as this provide important baseline epidemiological data regarding the risk of exposure to these neglected arboviruses. SSHV and JCV should be considered in the differential diagnosis for undiagnosed febrile and neuroinvasive illness during mosquito season, particularly when testing for common aetiologies is negative or inconclusive.

A-74 COVID-19 and Post-Acute Sequelae of SARS-CoV-2 Infection (PASC): Acute and Longitudinal Analysis of Anosmia and Ageusia with Delayed Sudden-Onset Neuropsychiatric Symptoms

Abstract Objective Patients with COVID-19 and PASC may exhibit chemosensory dysfunction associated with acute neuroinflammation from immune system overactivation (Uzunova, Pallanti, & Hollander, 2021). Neuropsychiatric disturbances in patients with no history of anxiety or depression have also been reported. These central nervous system manifestations of COVID-19 may be sequelae of trans-olfactory and infralimbic tract penetration (Speth et al., 2020). Methods Our case involved a 52-year-old, right-handed, American Indian female, who at three months post neuropsychological evaluation, was diagnosed with laboratory confirmed COVID-19 with onset of complete anosmia and ageusia. Two months later, a sudden-onset of panic and depression occurred with no precipitating event. All symptoms were documented daily until return of function. Results Pre-COVID-19 neuropsychological testing revealed findings consistent with ophthalmologic/vestibular migraine and ruled out dementia, and formal anxiety and depressive disorders. Post-COVID-19 neuropsychological analysis and follow-up revealed that anosmia and ageusia had largely resolved after 8-months, and that the delayed sudden-onset panic and depression also resolved within that same time period. Conclusions A paucity of data exists concerning COVID-19 and PASC anosmia and ageusia, and sudden-onset neuropsychiatric symptoms. Our case is unique since neuropsychological testing preceded the COVID-19 infection, which provided a baseline of functioning (e.g., Pocket Smell Test: 3/3 baseline and 0/3 acute COVID-19) and pre-morbid diagnostic specificity. The present case findings align with Cappali and Gatti (2021) whereby 91% of patients reported olfactory recovery, with 53% total recovery after 8-months. No other known reports simultaneously documented detailed recovery of anosmia, ageusia and delayed sudden-onset panic and depression, and COVID-19 antibody laboratory testing.

Neurological Manifestations of Coronavirus Disease 2019: A Comprehensive Review and Meta-Analysis of the First 6 Months of Pandemic Reporting

Background: There is growing evidence that SARS-Cov-2 infection is associated with severe neurological complications. Understanding the nature and prevalence of these neurologic manifestations is essential for identifying higher-risk patients and projecting demand for ongoing resource utilisation. This review and meta-analysis report the neurologic manifestations identified in hospitalised COVID-19 patients and provide a preliminary estimate of disease prevalence.Methods: MEDLINE, Embase and Scopus were searched for studies reporting the occurrence of neurological complications in hospitalised COVID-19 patients.Results: A total of 2,207 unique entries were identified and screened, among which 14 cohort studies and 53 case reports were included, reporting on a total of 8,577 patients. Central nervous system manifestations included ischemic stroke (n = 226), delirium (n = 79), intracranial haemorrhage (ICH, n = 57), meningoencephalitis (n = 13), seizures (n = 3), and acute demyelinating encephalitis (n = 2). Peripheral nervous system manifestations included Guillain-Barrè Syndrome (n = 21) and other peripheral neuropathies (n = 3). The pooled period prevalence of ischemic stroke from identified studies was 1.3% [95%CI: 0.9–1.8%, 102/7,715] in all hospitalised COVID-19 patients, and 2.8% [95%CI: 1.0–4.6%, 9/318] among COVID-19 patients admitted to ICU. The pooled prevalence of ICH was estimated at 0.4% [95%CI: 0–0.8%, 6/1,006].Conclusions: The COVID-19 pandemic exerts a substantial neurologic burden which may have residual effects on patients and healthcare systems for years. Low quality evidence impedes the ability to accurately predict the magnitude of this burden. Robust studies with standardised screening and case definitions are required to improve understanding of this disease and optimise treatment of individuals at higher risk for neurologic sequelae.

One-year results of a clinical trial of olipudase alfa enzyme replacement therapy in pediatric patients with acid sphingomyelinase deficiency

PurposeTo assess olipudase alfa enzyme replacement therapy for non–central nervous system manifestations of acid sphingomyelinase deficiency (ASMD) in children. MethodsThis phase 1/2, international, multicenter, open-label trial (ASCEND-Peds/NCT02292654) administered intravenous olipudase alfa every 2 weeks with intrapatient dose escalation to 3 mg/kg. Primary outcome was safety through week 64. Secondary outcomes included pharmacokinetics, spleen and liver volumes, lung diffusing capacity (DLCO), lipid profiles, and height through week 52. ResultsTwenty patients were enrolled: four adolescents (12–17 years), nine children (6–11 years), and seven infants/early child (1–5 years). Most adverse events were mild or moderate, including infusion-associated reactions (primarily urticaria, pyrexia, and/or vomiting) in 11 patients. Three patients had serious treatment-related events: one with transient asymptomatic alanine aminotransferase increases, another with urticaria and rash (antidrug antibody positive [ADA+]), and a third with an anaphylactic reaction (ADA+) who underwent desensitization and reached the 3 mg/kg maintenance dose. Mean splenomegaly and hepatomegaly improved by >40% (p < 0.0001). Mean % predicted DLCO improved by 32.9% (p = 0.0053) in patients able to perform the test. Lipid profiles and elevated liver transaminase levels normalized. Mean height Z-scores improved by 0.56 (p < 0.0001). ConclusionIn this study in children with chronic ASMD, olipudase alfa was generally well-tolerated with significant, comprehensive improvements in disease pathology across a range of clinically relevant endpoints.

Assessment of long non-coding RNA (THRIL and TMEVPG1) among Behçets' disease patients

BackgroundBehçet’s disease (BD) is a chronic inflammatory disorder with multifactorial cause. Long non-coding RNAs (lncRNAs) perform an essential role in gene regulation, and there is ongoing research in their contribution to autoimmune diseases. Aim of the workTo determine expression levels and diagnostic value of Theiler's Murine Encephalitis Virus Possible Gene1 (TMEVPG1) and TNFα and hnRNPL related immunoregulatory long non-coding RNA (THRIL) in BD, and to assess their role in the clinical characteristics of BD and disease activity. Patients and methodsStudy included 30 BD patients (12 females and 18 males) and 30 matched controls. Expression levels of TMEVPG1 and THRIL were detected by real-time polymerase chain reaction. ResultsThe mean age of patients was 37.5 ± 11.3 years and disease duration was 7.7 ± 6 years. Expression levels of TMEVPG1 and THRIL were upregulated significantly in serum of patients compared with controls (set as 1). TMEVPG1 fold change = 3.0 with interquartile range (0.2–546.9) (p < 0.0001), while THRIL fold change = 3.6 with interquartile range (0.1–112.3) (p < 0.0001). There was significant correlation between TMEVPG1 and THRIL (r = 0.48, p = 0.007). TMEVPG1 did not correlate with the clinical characteristics of patients, while THRIL correlated only with central nervous system manifestations. At a cut-off point of 1.3 the sensitivity of TMEVPG1 was 76.7%, while THRIL at 1.08 was 73.3% and specificity was 100% for both. ConclusionTMEVPG1 and THRIL were differentially expressed in the serum of BD patients and both had potential diagnostic value. Furthermore, an association between the expression level of THRIL and CNS symptoms was observed.

Clinical Characteristics, Molecular Background, and Survival of Egyptian Patients With Gaucher Disease Over a 20-Year Follow-up.

This study analyzes the general disease characteristics, impact of enzyme replacement therapy (ERT), and overall survival (OS) of 156 Egyptian patients with Gaucher disease (GD) enrolled on hormone replacement from 1998 to 2017. The mean age at diagnosis was 32.46±12.68 months. Anemia was noted at diagnosis in 50%, thrombocytopenia in 30.7%, severe splenomegaly in 58.7%, severe hepatomegaly in 11.9%, and skeletal findings were detected in 24.3% of the patients. The most prevalent GD type was type 3 (54.5%). Twenty-two of type 3 patients had no neurological manifestations at diagnosis, and 12 developed variable central nervous system manifestations during follow-up. The most common neurological features were limited eye movements, oculomotor apraxia, and squint. Of the 60 patients for whom genotypes were obtained, homozygous L444P was the most common (n=35/60, 58.3%). Treatment with ERT (imiglucerase) revealed significant improvements in blood indices, organ volumes, and growth parameters (P<0.05). Ten (11.7%) type 3 patients did not develop any neurological manifestations under ERT over 20 years. Mortality was 16%, and the 20-year OS was 73.3%. We conclude that in Egypt, type 3 is the most prevalent phenotype of GD, and homozygous L444P is the predominant GBA genotype of GD. Early age at diagnosis and treatment with ERT over 20 years revealed significant improvements in disease manifestations, with an OS of 73.3%.

Neurological and Head/Eyes/Ears/Nose/Throat Manifestations of COVID-19: A Systematic Review and Meta-Analysis.

Coronavirus disease 2019 (COVID-19) has been associated with various neurological and atypical head/eyes/ears/nose/throat (HEENT) manifestations. We sought to review the evidence for these manifestations. In this systematic review and meta-analysis, we compiled studies published until March 31, 2021 that examined non-respiratory HEENT, central, and peripheral nervous system presentations in COVID-19 patients. We included 477 studies for qualitative synthesis and 59 studies for meta-analyses. Anosmia, ageusia, and conjunctivitis may precede typical upper/lower respiratory symptoms. Central nervous system (CNS) manifestations include stroke and encephalopathy, potentially with brainstem or cranial nerve involvement. MRI studies support CNS para-/postinfectious etiologies, but direct neuroinvasion seems very rare, with few cases detecting Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) in the CNS. Peripheral nervous system (PNS) manifestations include muscle damage, Guillain-Barre syndrome (GBS), and its variants. There was moderate-to-high study heterogeneity and risk of bias. In random-effects meta-analyses, anosmia/ageusia was estimated to occur in 56% of COVID-19 patients (95% CI: 0.41-0.71, I2:99.9%), more commonly than in patients without COVID-19 (OR: 14.28, 95% CI: 8.39-24.29, I2: 49.0%). Neurological symptoms were estimated to occur in 36% of hospitalized patients (95% CI: 0.31-0.42, I2: 99.8%); ischemic stroke in 3% (95% CI: 0.03-0.04, I2: 99.2%), and GBS in 0.04% (0.033%-0.047%), more commonly than in patients without COVID-19 (OR[stroke]: 2.53, 95% CI: 1.16-5.50, I2: 76.4%; OR[GBS]: 3.43,1.15-10.25, I2: 89.1%). Current evidence is mostly from retrospective cohorts or series, largely in hospitalized or critically ill patients, not representative of typical community-dwelling patients. There remains a paucity of systematically gathered prospective data on neurological manifestations. Nevertheless, these findings support a high index of suspicion to identify HEENT/neurological presentations in patients with known COVID-19, and to test for COVID-19 in patients with such presentations at risk of infection.

Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants.

Neonatal lupus erythematosus (NLE) is a rare disease caused by passively transmitted autoantibodies from the mother. NLE is a multi-organ system disease characterized by cutaneous, cardiac, hematological, hepatobiliary, and neurological manifestations. This study aimed to review the various symptoms and clinical manifestations in young infants with NLE and their mothers. We conducted a retrospective review of medical records of patients with NLE who were both examined and treated at Pusan National University Children’s Hospital between January 2009 and December 2020 and their mothers. Twenty-seven patients with NLE comprising 13 male patients (48.1%) and 14 female patients (51.9%) were included. The most common symptom was rash (40.7%), followed by fever (25.9%), arrhythmia (14.8%), splenomegaly (11.1%), and intrauterine growth retardation (7.4%). Seven patients with fever had various organ system manifestations, including cutaneous (100%), hematological (71.4%), hepatobiliary (57.1%), and central nervous system (CNS; 28.6%) manifestations. Two of the febrile patients had aseptic meningitis. Cutaneous, cardiac, hematological, hepatobiliary, and CNS involvement were noted in 44.4%, 18.5%, 51.9%, 40.7%, and 22.2% of the patients, respectively. Systemic lupus erythematosus (SLE) was the most common maternal disease (14/27, 51.9%). Ten mothers (37.0%) had not been diagnosed with any autoimmune disease until their babies were diagnosed. Among them, three were subsequently diagnosed with SLE, five were diagnosed with the Sjögren’s syndrome, and two of them still had no known diagnosis of any autoimmune disorder. Fever is a common symptom of NLE; thus, when there is no clear focus of fever in infants, NLE needs to be considered, especially in cases with skin rashes.

Neurological Manifestations of Patients Infected with COVID-19: A Systematic Review

In the light of the recent evidence, neurological manifestations have been reported in patients infected with Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2). To shed light on a clear-cut explanation of neurological complications followed by SARS-CoV-2, a systematic analysis was reviewed. To this point, general medical databases, such as PubMed, Scopus, and Google Scholar from January 1, 2020, to September 20, 2020, were systematically searched to find related published articles. Through the search strategy, 93 studies for qualitative synthesis were included. Finally, the available data of 8,753 hospitalized patients with coronavirus disease 2019 (COVID-19) showed that the most prevalent Central Nervous System (CNS) manifestations of COVID-19 were headache (1137 cases, 18%) and dizziness (142 cases, 2%). On the other hand, some peripheral nervous system (PNS) symptoms were muscle pain (3300 cases, 53%), hypogeusia (646 cases, 11%), anosmia (545 cases, 9%), and hyposmia (379 cases, 6%). Furthermore, a small proportion of patients had ageusia (74 cases, 1%), cerebrovascular disorders (66 cases, 0.75%), and impaired consciousness (21 cases, 0.2%). Besides, some neurological features, such as encephalitis, neuralgia, ataxia, Guillain- Barre syndrome, miller fisher syndrome, intracerebral hemorrhage, polyneuritis cranialis, and dystonic posture were reported in the literature as case reports and case series. Conclusion: Taken together, data from our systematic analysis indicated that neurological manifestations in both CNS and PNS were reported in COVID-19 patients. Further observational research in this field with more participants are needed to detect the specific risk concerning neurological complications in COVID-19 infection.

COVID-19 and Central Nervous System Manifestations: A Systemic review on Therapeutic Outcome

A novel caused the outbreak of coronavirus disease (COVID-19) has spread to Wuhan city of China and has become a global health issue and severe concern of the international community. Elderly patients with comorbidities, such as hypertension, diabetes mellitus, heart and lung disease, are more prone to COVID-19 Infection. COVID-19 and neurological disorders are two conditions with a well-documented association recently. Studies have shown that Infection with COVID-19 can alter the clinical spectrum of manifested neurological disorders and plays a vital role as long-term consequences in the development of diseases in the future. The SARS-CoV-2 virus reaches the brain either by an olfactory or by a hematogenous route pathway. Angiotensin-converting enzyme-2 (ACE-2) receptors, present in cerebral vessel endothelial cells of a cerebral vessel, are a potential viral entry point. Several cases of neurological disease have been identified recently, including Alzheimer's disease, Multiple sclerosis, and Parkinson's disease in COVID-19 patients, and a post-infectious immune-mediated inflammatory pathway has been held responsible for this. In this study, we try to explore the effect of COVID-19 on central nervous system damage and treatment of these manifestations. COVID-19 pandemic spreads to different parts of the world, the spectrum of neurological complications can expand further.

Signs and Symptoms of Central Nervous System Involvement and Their Pathogenesis in COVID-19 According to The Clinical Data (Review)

Detailed clinical assessment of the central nervous system involvement in SARS-CoV-2 infection is relevant due to the low specificity of neurological manifestations, the complexity of evaluation of patient complaints, reduced awareness of the existing spectrum of neurological manifestations of COVID-19, as well as low yield of the neurological imaging.The aim. To reveal the patterns of central nervous system involvement in COVID-19 and its pathogenesis based on clinical data.Among more than 200 primary literature sources from various databases (Scopus, Web of Science, RSCI, etc.), 80 sources were selected for evaluation, of them 72 were published in the recent years (2016-2020). The criteria for exclusion of sources were low relevance and outdated information.The clinical manifestations of central nervous system involvement in COVID-19 include smell (5-98% of cases) and taste disorders (6-89%), dysphonia (28%), dysphagia (19%), consciousness disorders (3-53%), headache (0-70%), dizziness (0-20%), and, in less than 3% of cases, visual impairment, hearing impairment, ataxia, seizures, stroke. Analysis of the literature data revealed the following significant mechanisms of the effects of highly contagious coronaviruses (including SARS-CoV-2) on the central nervous system: neurodegeneration (including cytokine- induced); cerebral thrombosis and thromboembolism; damage to the neurovascular unit; immune-mediated damage of nervous tissue, resulting in infection and allergy-induced demyelination.The neurological signs and symptoms seen in COVID-19 such as headache, dizziness, impaired smell and taste, altered level of consciousness, bulbar disorders (dysphagia, dysphonia) have been examined. Accordingly, we discussed the possible routes of SARS-CoV-2 entry into the central nervous system and the mechanisms of nervous tissue damage.Based on the literature analysis, a high frequency and variability of central nervous system manifestations of COVID-19 were revealed, and an important role of vascular brain damage and neurodegeneration in the pathogenesis of COVID-19 was highlighted.

Neurological Manifestations of COVID-19

The year 2020 started with the news of a novel coronavirus, severe acute respiratory syndrome corona virus 2(SARS-CoV2), induced disease in China which was termed as coronavirus disease 2019 (COVID-19). This viralinfection soon became pandemic and affected millions of people all over the world. The virus preferentiallyaffects respiratory system causing dry cough and fever, but has the tendency to spread to different organs in the body leading to multiple organ failure. Recent evidences show that corona virus can invade nervous system and damage it. This review is based on different articles and case reports that provide an evidence of neuro-virulent nature of COVID-19 and its consequences. The neuro-invasive property of the virus is divided into threecategories i) Central Nervous System (CNS) manifestations, ii) Peripheral Nervous System (PNS) manifestationsand iii) Skeletal Muscle damage. Headache and dizziness were observed to be common symptoms for CNS,whereas loss of smell and taste for PNS damage due to COVID-19. The aim of this review is, to develop anunderstanding of the devastating effects of COVID-19 on nervous system for the early recognition of virusinduced damage. This information can be used for the development of better therapeutic strategies.

NCoV-2019 infection induced neurological outcome and manifestation, linking its historical ancestor SARS-CoV and MERS-CoV: a systematic review and meta-analysis

The first systematic review and meta-analysis to help clinician to identify early signs and symptoms of neurological manifestation in COVID-19 positive patients which will further help in early management of patients. Present systematic review and meta-analysis aimed to discuss the prevalence of neurological involvement of the 2019-nCoV patients and assess the symptomatic trend of events as compared to the 2002 “SARS” and 2012 “MERS” pandemics.The articles were systematically screened through several search engine and databases. The articles published or in preprint were included in the study till 15th May 2020. The systematic review done as per the published literatures which included 31 cross sectional, observational studies and case reports which revealed neurological signs and symptoms in SARS-COV-2 disease. For meta-analysis, we included 09 observational and cross-sectional studies which included COVID-19 positive patients and assessed the predominance of various neurological signs and symptoms in COVID-19 patients with relation to SARS-2002 and MERS-2012. Data was analyzed by using the “MedCalc” Statistical Software version 19.2.6 and reported as pooled prevalence. Standard I2 test was used to analyze the heterogeneity. We have collected and screened about a total 2615articles, finally we have included 31articles for the systematic review and 09 for meta-analysis as per the inclusion/exclusion criteria. The analysis was made as per the prevalence rate of neurological symptoms in COVID-19 positive patients. The cumulative neurological outcome of SARS-2002 and MERS-2012 was assessed to get the trends which was further tried to correlate the events with the current pandemic. During the analysis severity and outcome of neurological manifestations range from simple headache to vague non-focal complaints to severe neurologic impairment associated with seizure or meningitis. Central and peripheral nervous system (CNS/PNS) manifestations were seen during the SARS-2002, MERS-2012 and COVID-19. However, none of the publication had primary or secondary objectives of searching neurological manifestations in the COVID-19 patients and the pathogenic mechanism which will subsequently strengthen the importance to start more prospective clinical trials. The prevalence of neurological signs and symptoms were taken as primary objective. Thereafter, the prevalence of each CNS/PNS symptoms was categorized and their prevalence studied. The selection of Bagheri et al., 2020 may be discussed because they have done the cross-sectional study with the neurological finding and correlated the data with prevalence of the COVID-19 positive patients. The proportion of patients presenting with neurological outcome and clinical/PCR positivity were done. We had searched and followed all the possible online/web source, still the data collection process may remain a limitation of work due to addition of several publications on COVID-19 every day. Due to lack of data of SARS-CoV and MERS-CoV, we have included the case reports, MERS and COVID-19 in CNS/PNS manifestations.

Rare case of recurrent stroke in a patient with eosinophilic granulomatosis with polyangiitis: a case report

BackgroundCentral nervous system involvement is rarely described in eosinophilic granulomatosis with polyangiitis (EGPA) and occurs in 5–9% of patients. Among central nervous system manifestations, cerebral infarctions are the most common. To the best of our knowledge, a recurrent stroke in patients with EGPA without cardiac risk factors during maintenance therapy so far has not been described.Case presentationA previously healthy 57-year-old female during the course of 1 year developed asthma, sinusitis, polyneuropathy, muscle weakness, and rash followed by fatigue, myalgia, arthralgia, and fever. After an initial diagnostic evaluation, elevated values of eosinophils, liver enzymes, creatine kinase, lactate dehydrogenase, and inflammatory markers (sedimentation rate and C-reactive protein) were found, and renal impairment was detected. On the third day of hospitalization, she developed left-sided hemiparesis due to an ischemic stroke in the right basal ganglia. She has been diagnosed with EGPA, and oral corticosteroid, immunosuppressive, and antiplatelet therapy were applied. Despite potent treatment and initial recovery, a few weeks later, she developed recurrent ischemic stroke in the left hemisphere and pulmonary embolism as rare and potentially severe complications of EGPA. Assuming that complete disease remission had not been established previously, oral prednisone was initially substituted with intravenous methylprednisolone pulses. During follow-up, immunosuppressive therapy was slowly discontinued, oral corticosteroid therapy was reduced to a maintenance dose, and thromboembolic events were well controlled by oral anticoagulant therapy.ConclusionAnticoagulant therapy, in addition to immunosuppressive maintenance therapy, should be considered in any EGPA patient who has had an ischemic stroke even without cardiac risk factors.

Transient Horner syndrome associated with COVID-19: A case report.

The Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2), the virus that causes coronavirus disease 2019 (COVID-19), has spread as a global pandemic with significant morbidity and mortality. As the prevalence of COVID-19 has risen, so has the diversity of its clinical presentation. SARS-CoV-2 is considered to have neuroinvasive and neurotropic qualities that can lead to central and peripheral nervous system manifestations. We describe a 65-year-old woman who developed new-onset unilateral ptosis and mitosis following a diagnosis of COVID-19. To our knowledge, this is the first reported case describing transient Horner syndrome in association with COVID-19.

A case of rheumatoid meningitis with elevated antibody titer indices of anti‐cyclic citrullinated peptide antibody and anti‐galactose‐deficient IgG antibody

AbstractRheumatoid meningitis (RM) is a rare central nervous system manifestation of rheumatoid arthritis (RA). A meningeal biopsy is useful for diagnosis but is invasive and not very sensitive. We report about a patient with RM who was diagnosed using indices of anti‐cyclic citrullinated peptide (CCP) antibody and anti‐galactose‐deficient IgG antibody (carbohydrate in rheumatoid factor [CARF]) and who had refused to undergo biopsy. The patient's antibody titer indices of anti‐CCP antibody and CARF were 21.01 and 4.44, respectively. The patient's symptoms and abnormalities on brain magnetic resonance imaging improved after steroid therapy. Antibody titer indices of the anti‐CCP antibody and CARF can be used to diagnose RM in cases where biopsy is unavailable.

Neuroimaging findings in rheumatologic disorders

Patients with rheumatological diseases may present with neurological manifestations of peripheral and/or central nervous system (CNS). Symptoms may be related to underlying rheumatological disease or CNS effects of immune-modulating drugs. Early diagnosis and therapy may help prevent serious complications. Magnetic resonance imaging (MRI), given its excellent soft tissue details, is the preferred imaging modality when evaluating patients with rheumatological disease and suspected CNS involvement. We present a review of the neuroimaging manifestations of various rheumatic diseases with emphasis on the imaging findings on MRI.

Mixed Nervous System Involvement in a Child with Multisystem Inflammatory Syndrome- A Rare Presentation of SARS-CoV-2

Multi-system inflammatory syndrome (MIS-C) is a rare but severe complication of Covid-19 infection. The clinical presentation ranges from a mild course of systemic inflammatory response to a life threatening hyper inflammatory shock and multi organ dysfunction. Central nervous system presentation is seen in acute Covid-19 infection in adults but is rarely seen in children. We describe a case of MIS-C in a child who was admitted to our Pediatric Intensive Care Unit with features of hyper inflammatory syndrome and mixed central and peripheral nervous system manifestations.

Histiocytosis and the nervous system: from diagnosis to targeted therapies.

Histiocytoses are heterogeneous hematopoietic diseases characterized by the accumulation of CD68(+) cells with various admixed inflammatory infiltrates. The identification of the pivotal role of the mitogen-activated protein kinase (MAPK) pathway has opened new avenues of research and therapeutic approaches. We review the neurologic manifestations of 3 histiocytic disorders with frequent involvement of the brain and spine: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), and Rosai-Dorfman-Destombes disease (RDD). Central nervous system (CNS) manifestations occur in 10%-25% of LCH cases, with both tumorous or neurodegenerative forms. These subtypes differ by clinical and radiological presentation, pathogenesis, and prognosis. Tumorous or degenerative neurologic involvement occurs in 30%-40% of ECD patients and affects the hypothalamic-pituitary axis, meninges, and brain parenchyma. RDD lesions are typically tumorous with meningeal or parenchymal masses with strong contrast enhancement. Unlike LCH and ECD, neurodegenerative lesions or syndromes have not been described with RDD. Familiarity with principles of evaluation and treatment both shared among and distinct to each of these 3 diseases is critical for effective management. Refractory or disabling neurohistiocytic involvement should prompt the consideration for use of targeted kinase inhibitor therapies.